l**t
发帖数: 321 | 1 父亲这两个星期都在医院做各种各样的检查，今天医生初步判断是运动神经元病。一早
收到 这个消息不能平静下来。目前唯一可用的药就是力入太（Rilutek），不能治疗，
只是延长几个月的寿命。
父亲才六十多岁，身体很好，生活方式健康，也喜欢运动，实在很难接受这样的病和父
亲联系起来。一直以为父亲会长寿，以为还有很多的时间和机会孝敬父亲。
知道置顶文章中已经说过处方药的问题，还是抱着一线希望有人能告诉我如何买到
AVENTIS生产的Rilutek （riluzole）。 万分感谢！ | f****o
发帖数: 2770 | 2 国内没有进口药可以买么？去北京上海大地方问问看啊
美国你是买不到的
Amyotrophic lateral sclerosis病末期病人肌肉全部瘫痪动都不能动，话也不会说，
呼吸也不能自主，你先去学习下如果照顾ALS末期病人，减轻你父亲未来的痛苦吧。 | l**t
发帖数: 321 | 3 要去北京最后确诊的，估计确诊以后应该可以买到。我想买一些算是尽自己的心吧。
LS了解这个病吗？这一点也是这个病最可怕的地方，真是太痛苦了。。。。 | f****o
发帖数: 2770 | 4 Background information
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is
a motor neuron disease that attacks motor nerve cells in both the brain and
spinal cord. Motor nerves control muscles that can be mentally directed,
including those for the face, mouth, arms, legs, and breathing. ALS does not
typically affect muscles that are not mentally controlled, such as external
sphincters that hold stool in the bowel and urine in the bladder, muscles
that control eye movement, sexual function, internal organs and the five
senses. This devastating disease has unknown causes and can strike anyone at
any age; however, it is not contagious. There are approximately 6,000 new
diagnoses of ALS each year and as many as 30,000 Americans living with ALS
at any given time.
Progression
ALS is a progressive disorder that makes people become weaker over time
gradually. Each patient progresses at a different rate and the muscles being
affected varies from patient to patient as well. Not everyone has the same
symptoms and nor do they develop all the symptoms listed below. However,
patients will lose control over most of their voluntary muscles eventually.
ALS Symptoms:
Muscle weakness and wasting
“Charleyhorse” type muscle cramps, twitches under the skin
Swallowing difficulty and nutrition deficiency
Weight loss due to muscle wasting and poor nutrition intake
Exaggerated laughing or crying and depression
Stiffness and poor coordination
Fatigue and exhaustion
Urinary urgency (but not incontinence)
Swelling of hands and feet
Excessive saliva, thick phlegm and postnasal drip
Constipation
Speech difficulty
Breathing difficulty due to weakness of the diaphragm
Referral to hospice
Patients with ALS can be referred to hospice when there is rapid disease
progression that leads to life-threatening complications, critically
impaired breathing capacity or nutrition status. Hospice care focuses on
symptom relief for patients as well as providing tremendous support to
caregivers.
Medications to Treat ALS
Unfortunately, there is no cure for ALS. Riluzole (Rilutek®), a
glutamate antagonist, is the only FDA approved medication to slow down the
disease progression. It is most useful in those with early and less severe
disease. When someone enters the end stage of the disease, riluzole is
usually stopped since the patient has already received maximal benefit from
the medication.
Medications/Devices to Help with Symptoms Associated with ALS
Symptoms management is the key when providing comfort care to ALS patients
at the end stage of life and should be patient specific. Some end of life
symptoms and management strategies are listed below to help you better
understand what to expect when taking care of ALS patients.
1. Breathing Difficulty: This is one of the most critical issues for the
majority of patients with ALS. Physicians may suggest using mechanical
assistance to help patients breathe. Options include non-invasive positive
pressure ventilation, In/Exsufflator and mechanical ventilator. Please feel
free to discuss this with the medical team to determine the best option for
your loved ones.
2. Nutrition and Hydration: Obtaining enough nutrition becomes more
challenging due to not being able to swallow. A feeding tube can be placed
to assist with food and fluid intake if necessary.
3. Speech and Communication Difficulty: Patients can lose their ability
to talk to caregivers. There are many different assistive devices available
to help ALS patients. You can ask the medical team about using one of those
devices to aid in communication.
4. Depression and Uncontrolled Emotions: Behavioral distress is common
among ALS patients especially at the end stage of life. Antidepressants can
be used to alleviate those symptoms.
5. Pain: There is a misperception that pain is not a significant problem
in ALS patients; however, more than 70% of ALS patients in hospice report
pain complaints. Pain should be assessed frequently to ensure comfort of the
patients.
Non-pharmacological interventions: range-of-motion exercises,
frequent repositioning, therapeutic mattresses, relaxation and diversion are
worth a try.
For cramps and spasticity: quinine (Qualaquin®), diazepam (
Valium®), baclofen (Lioresal®), tizanidine (Zanaflex®), and
gabapentin (Neurontin®) are effective.
For musculoskeletal discomfort: aspirin, acetaminophen (Tylenol&
#174;) and non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen
(Advil® or Motrin®) and naproxen (Aleve®) can be used.
6. Insomnia: Insomnia in ALS patients is largely contributed to breathing
difficulty, depression and in late ALS, nocturnal pain. Sedating
antidepressants such as amitriptyline (Elavil®), trazodone (Desyrel&#
174;) and longer acting sedative/hypnotic agents, such as temazepam (
Restoril®) are commonly used to aid sleep.
Discontinuing Medications
The hospice physician or nurse may recommend discontinuing medications for
several reasons:
Undesired side effects
Medications no longer beneficial at the end stage of life
Medications cannot be administered via feeding tube
For More Information
Please contact the hospice care team for more information about amyotrophic
lateral sclerosis. The resources listed below contain more information on
the disease and provide patient/family support.
• ALS Association-Fighting Lou Gehrig’s Disease at: www.alsa.org
• Les Turner ALS Foundation at: www.lesturnerals.org
• Muscular Dystrophy Association at: www.als-mda.org
• Project ALS at: www.projectals.org
• ALS Therapy Development Institute at: www.als.net
• International Alliance of ALS/MND Associations at: www.
alsmndalliance.org
• National ALS Registry at: www.cdc.gov/als/
• World Federation of Neurology ALS at: www.wfnals.org | l*h
发帖数: 4124 | 5 "要去北京最后确诊的"
ALS can be diagnosed by history and physical and neurological examination.
advanced imaging and lab studies are rarely needed for its diagnosis if the
history and signs are clear. it is strange that most "neurologists" in china
don't know how to make the diagnosis. most "neurologists" in china don't
know how to diagnose multiple sclerosis either.
【在 l**t 的大作中提到】
: 要去北京最后确诊的，估计确诊以后应该可以买到。我想买一些算是尽自己的心吧。
: LS了解这个病吗？这一点也是这个病最可怕的地方，真是太痛苦了。。。。
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